In limbo

So I thought I would write a quick entry because it seems that I only write when I have a ton of stuff to update, and by the time I'm done it's all information and not a lot of substance.

I've been thinking a lot since we got home from the hospital on Monday. What my life is going to consist of for the indefinite future. How much things can change in a short period of time. (Can you believe it hasn't even been three months since Eddy's first seizure? It feels like about three years.) How it's so easy to take things for granted when life is easy and everything is going well. How, if my life gets back to "normal" (whatever that is), can I make sure that I no longer take peace and normalcy for granted?

Eddy has been doing great since we got home from the hospital. No seizures in eleven days, his physical development is continuing to come along. He's crawling faster, pulling up and getting into a sitting position easier, and cruising along furniture more every day. He seems to have gotten used to his new seizure medication and seems to be increasingly more "with it." He's starting to make more sounds, although verbally he has a long way to go. One of the things that I'm the most scared of is Eddy being non-verbal. A lot of the genetic disorders and metabolic disorders that have seizures and developmental delays as symptoms result in non-verbal future outcomes. I know that things could be worse than that, but I really want Eddy to talk. Badly. His speech therapy was just increased to 8x/month (it was 4x/month) and I'm hoping that helps. If he just started talking, I would feel so much better about his future outcome.

I'm also really worried because, looking at past experience with Eddy, he seems to do really well after starting a new seizure medication, but a short time later, it seems to stop working and he starts having seizures consistently once again. I'm really hoping that doesn't happen with the phenobarbital, but I know it's a possibility.

I got a phone call yesterday afternoon from Cleveland Clinic. I had called the previous day asking about the results of Eddy's PET scan. A nurse called me back and said, "His PET shows mild assymetry with left temporal glucose being mildly low, possibly reflecting recent seizures." Um, what? I like to think that I'm pretty smart, or at least I can do a good google search when I don't understand something; but an hour's worth of research resulted in me having no idea what the heck the nurse was talking about and thinking Eddy might have Alzheimers Disease. (If you google that exact description, a few pages about Alzheimer's comes up.) I asked the nurse what that meant and he said, "I don't know, Dr. Gupta spoke with Dr. Weinstock and he will discuss the results with you once he receives the written report." Of course, I immediately called Dr. Weinstock's office and am currently waiting for them to call me back about the results.

If there are any doctors out there with experience in brain PET scans and can kindly explain to me the results of Eddy's scan before Monday, I would greatly appreciate it. :)

I've been a little discouraged lately because I feel like I'm chained to my house. The doctor in Cleveland said that he believes Eddy is the type of kid who will get sick easily (hence the double ear infection two weeks ago when I was trying hard to keep him healthy), and when he's sick he will get seizures. So he advised us to do what we need to to keep Eddy healthy. This basically means that I need to keep Eddy away from people who spread germs, and these people are usually little kids. And all of the places I would take Eddy to socialize (play group, the YMCA, etc.) have little kids around. I know that I will (hopefully) be less paranoid in the future, but for now I'm keeping Eddy away from as many germs as I can. And that means we are a lot more isolated than I would like to be. But this, too, shall pass I'm sure, and I just have to keep reminding myself that. It's much better to be "chained" to my spacious new house than the hospital! (It could always be worse...another thing I regularly remind myself of.)


Ed and I got a card today from the great people at Olean High School, where I taught math for two years before resigning this past year to stay home with Eddy. They included a very generous gift certificate and gas gift card. The card was signed by dozens of faculty, staff, and students I used to work with. It was so touching to know that so many people are thinking of us and praying for us. Thank you so much Olean High - you are so kind and generous and we really really appreciate it.

And thank you to everyone who has continued keeping Eddy in your prayers and thoughts. We are so lucky to have all of you in our lives.

Cleveland Clinic

So much has happened since my last post that I don't even know where to begin (and I don't have a ton of time so I'm going to attempt to consolidate.) Last Sunday, October 14, Eddy woke up and had his typical tonic/clonic seizure that he had been having around 7 am. We were obviously upset and unhappy, but we weren't surprised. He had been having this seizure every five days or so, and it had been six days since he had it last. I took him downstairs to change his diaper and give him his seizure meds, and as I was changing his diaper, he had another seizure. This absolutely terrified me because 1.) He had never had two seizures that close together before, and 2.) He was awake and all of his other seizures had been when he was sleeping. The second seizure was a tonic seizure where his arms come up and go rigid for 10-15 seconds, and his eyes kind of bug out of his head. I ran upstairs to tell Ed, and he couldn't believe he had another seizure. He came downstairs with me to help me give Eddy his medicine, and he had another tonic seizure when he was in his high chair. At this point I basically freaked out and called the neurologist on call at Children's. When I was waiting for her to call me back, Eddy had yet another seizure. She called me a few minutes later, and as I was talking to her, he had his fifth seizure. She advised me to give him Diastat, a rectal emergency seizure medicine that is supposed to stop bad seizures. She also told me to call 911 and get him to the ER as soon as possible.

We gave him the Diastat as soon as we got off the phone with her, but it did nothing, and he continued having seizures every 3 to 4 minutes. By the time the ambulance came, he had had maybe eight or nine seizures. By the time we got to the hospital, Eddy had had about twelve seizures. All of them, other than the first one, were the tonic ones where he went rigid for anywhere from 5-20 seconds.

At the hospital, he was having a seizure every 4 to 5 minutes. The ER doctor in Olean called Buffalo Children's to see what they wanted him to do, and they told him to have the pharmacy make an IV solution of some derivation of phenobarbitol (I can't remember what they said the drug was called at this point). They were hoping this would stop the seizures. We waited for the pharmacy to make it, and he continued to have seizures. Finally, they hooked it up to his IV (which took about four pokes to get in, poor kid), but he continued to have a seizure every five minutes. It took about twenty minutes of the medicine to be in his system for him to stop having seizures. I was absolutely terrified at this point, thinking that nothing would stop them. He had had TWENTY seizures in less than a three hour period!

We were waiting for Eddy to be transported to Buffalo, and they were planning on taking him by helicopter. The helicopter took off with the STAT team from Buffalo, but they had to turn around because of the weather. So we had to wait for them to get back to Children's, get the ambulance ready, and drive down to Olean. While we were waiting, Eddy had a few more short seizures. Hours later, Eddy was being taken by ambulance to Children's. When we got there, we spent several more hours waiting in the ER, and he had a couple more seizures there, making the count about 25. We were finally admitted around 6 pm, eleven hours after all this craziness ensued. When he was in his hospital room, Eddy was started on Ativan, another drug to stop seizures. He had been given so many drugs over the course of the day that he was almost completely sedated and out of it. Ed and I went to dinner to get out of the hospital and try to regain some sanity, and my parents watched Eddy. While we were at dinner, he had a few more seizures, but they were different than any he's ever had; he just went limp and his head dropped a few times. It was hard to tell if they were seizures or if they were just from him being sedated and exhausted, but he was hooked up to an EEG at this point, and it was verified that he had four seizures on the EEG. The interesting thing was that the seizures were not infantile spasms, as we thought they were, but they were focal seizures coming from one area on the left side of his brain. This is much better than infantile spasms, which are more generalized and come from deep inside the brain. Previously, Eddy's EEG showed more generalized seizures, meaning they came from both sides and not one specific area. Now it appeared that his seizure type had changed, at least the four that were caught.

In the two and a half days he was hooked up to the EEG in Buffalo, five seizures were caught (the last one being on Monday), and they were all focal. They said that actually his EEG looked better than it had previously, which was not what we were expecting after everything that had happened. He was still having abnormal waves, but the waves that were reminiscent of hypsarrythmia were gone.

To make matters more confusing, Eddy's neurologist was out of the country on a family emergency. Because we had no idea what was going on, and his neurologist was gone, we decided to just see if we could get Eddy transported to the Cleveland Clinic. We had looked into taking him there on an out-patient visit, but now we wanted him to be transported there immediately to see if they could give us some more answers. Luckily my parents had taken the entire week off of work and were at the hospital every day (Thanks mom and dad, you are amazing!), and so I was able to track down the neurologist on call and make tons of phone calls to try to make this transfer happen. I just showed up in the Neurology Clinic, which was next door to the hospital, and I asked to talk to the nurse and the doctor. The doctor on call was great, and he was supportive of the transfer. He called the neurologist on call at the Cleveland Clinic, and he agreed to accept Eddy. Then he called the insurance company and had several talks with them to convince them to approve this transfer. After all of this, the medical director denied it, saying since Eddy wasn't in the ICU, it wasn't necessary. Then, five hours after I got the call saying it was denied, I got another call from the insurance company saying it was approved. It turns out the first medical director failed to put in the paper work, and the nurse had to ask a second medical director to review Eddy's case. He, in turn, immediately approved it. It's comforting to know that something as important as this isn't at all subjective, isn't it?

So this was Wednesday afternoon, and we were told that we had to wait until Cleveland had a bed available for him to be transferred. On Thursday, Eddy had an MRI, and this came back normal once again (He had had an MRI back in August but they wanted to see if his seizures had changed his MRI at all.) Thursday afternoon we were told that we would be transferred to Cleveland the next day. Friday around noon, the STAT team came and Eddy and I were taken by ambulance to Cleveland. We were thrilled that it was finally happening, and we knew that it was a result of many prayers. We were hoping for some more answers, but we also knew that Eddy's case was very atypical, not clear-cut, and most likely would continue to be nebulous in Cleveland. Even if we could get a few more answers, though, we would be happy.

Eddy was hooked up to another EEG as soon as we got to Cleveland, on Friday afternoon. From then until now (Monday afternoon), he has had no seizures. I forgot to mention that Eddy was started on Phenobarbital on Tuesday. I was very opposed to the Phenobarb before any of this happened (it delays development, which is not what Eddy needs!), but knowing that Dr. Weinstock, Dr. Langan, and Dr. Gupta (the neurologist in Clevelend who was consulted by the other two neurologists) all agreed it could help Eddy, we decided to go for it. It has helped him up to this point, because as I just stated, he hasn't had any seizures since Monday! We're hoping it continues to help.

We have met with Dr. Ajay Gupta, a world-class neurologist here at the Cleveland Clinic, three times since we've been here, Saturday, Sunday, and today. He is very impressive, patient, knowledgeable, and has answered lots and lots of our questions. Dr. Gupta reviewed all of Eddy's records and looked at his EEG from this visit. Let me attempt to summarize everything he's said...

Eddy is an atypical case, but he said he is atypical in a good way. He does not think Eddy has infantile spasms right now (probably the best news we heard from this whole trip!!) The brain waves that were reminiscent of hypsarrythmia are gone, and his EEG actually looks pretty good right now. He said that Eddy still has some slow waves on the left side, but his EEG looks better than it did. This could be a result of the phenobarb or it could be his brain maturing or it could be his seizures changing. His EEG has changed so much already that I know not to get too optimistic because a new medicine can clean up the EEG, but after he gets used to it, the next EEG can look worse once again. He said that Eddy definitely has epilepsy, and epilepsy is always very unpredictable. Even if two kids have the same seizure disorder and similar EEGs, their prognosis and outlook can be completely different.

Since Ed and I are numbers people, we appreciated that Dr. Gupta gave us some percentages. He said that Eddy had a 33% chance of outgrowing the seizures when he gets older. That's the best case scenario and the one we are really hoping for. He said that Eddy had a 33% chance of having a disorder that causes epilepsy for his entire life. One possibility is Dravet Syndrome, which is a genetic disorder that causes seizures and developmental and cognitive delays. It is a spectrum disorder, so even if he has it, there's no way of knowing where he will be developmentally in ten years. This is obviously something we're hoping he doesn't have. They did the test in Buffalo on October 5, and they said it can take six to eight weeks for the results to come back (Only two facilities in the country do the test for Dravet.)

Dr. Gupta ordered a PET Scan, which Eddy had this morning, to see if he has cortical dysplasia or some other abnormality with his brain that is so small that it wasn't found on the MRIs. The reason he did this is because Eddy's seizures are now coming from one spot on the left side of his brain, and this could be caused by cortical dysplasia.

We are about to be discharged from the Cleveland Clinic so I'm running out of time, but I wanted to update everyone who has been asking. Although we still don't know exactly where Eddy will be or what will happen with him, this has been a beneficial trip. We understand now that Eddy's epilepsy could go in any direction, and we're hoping that with the right combination of drugs, we will be able to control it (and hopefully he will outgrow it). So now we are waiting on the results of the PET scan, which we should hear something about in a couple of days, and the Dravet test, which we should hear something about in four or five weeks. Thank you so much for all of your prayers and encouraging words.

Waiting...Again.

If God appeared to me right now, I think I would say, "Wow, God, I don't understand why you're letting this happen and how any this can be Your will."

Since my last post almost a week ago, we increased Eddy's Keppra and waited to see what would happen. Well, Eddy had another seizure Monday afternoon during his nap, and it was the same as the previous two seizures - a spasm that turned into a tonic seizure (he goes rigid), then it turns into a tonic/clonic seizure (starts shaking after he goes rigid). I called Dr. Weinstock, his neurologist, and he said that we needed to start him on a treatment, and he recommended prednisolone. This is a steroid, similar to ACTH but not as harsh. It is administered orally, as opposed to having to give Eddy a shot every day. It also suppresses the immune system, but not quite as bad as ACTH does. It still has quite a few bad side effects, but we were happy to have finally made a decision. He called in the prednisolone, we picked it up, and we were ready to start it on Wednesday.

Then Wednesday morning at 2 am, Eddy woke up with a horrible runny nose and cough. If you have read my previous blog posts, you may be thinking "Hmm, am I sensing some déjà vu? This is sounding familiar." Yes; the last time we were going to start a treatment (ACTH), Ed got sick right before we were going to start it. The funny thing is, Ed (my husband) never gets sick, and Eddy hasn't been sick in months (Other than having seizures).

So I called Dr. Weinstock's office again yesterday morning, and he agreed that we need to wait until Eddy is better to start the prednisolone. His immune system, since it will be suppressed, has to be in top condition whenever we start the steroid.

Is God trying to tell us something?? Isn't this a little coincidental?? Both times we were going to start a treatment, someone gets sick right before and we have to wait.

So Eddy was still sick this morning, and his cough had gotten worse. Also, he developed a rash on his face. I took him to his pediatrician this afternoon, and it turns out Eddy has a double ear infection and eczema! And on top of that, his bottom molars are coming in! I just don't understand how God can let all of this happen to our poor little boy. It seems like everything is hitting him at the same time, and our typically sweet, happy baby is absolutely miserable. Which means, as all you parents out there know, that his mommy and daddy are not exactly the happiest of campers either.

Now that we are forced to wait again, I decided to try to make this a productive waiting game and look for a second opinion to see if we can find someone who has seen a case like this before. Initially, we just wanted to get Eddy started on a treatment to make him better and didn't want to take the time to get into another doctor. But now we decided a second opinion might be a good idea.

Don't get me wrong, I respect Dr. Weinstock tremendously. He is a great neurologist, and he definitely has Eddy's best interest in mind. But Buffalo Children's, comparatively, is a small hospital and doesn't even rank in the top fifty of the US News and World Reports Pediatric Neurology programs. So I contacted the #1 rated program (Boston Children's Hospital) and the #3 program (Cleveland Clinic). We are going to send Eddy's records to both facilities, and ideally we would like a second opinion over the phone (especially from Boston). We would be willing to travel to Cleveland (actually we would be willing to travel anywhere, if it could help Eddy) because Cleveland is only 3 and a half hours away. I spent some time today researching the Pediatric Epilepsy Center at Cleveland Clinic and I am very impressed. Their technology and experience is top-of-the-line, and I am encouraged by the thought of someone there potentially having seen a case like Eddy's before and knowing what to do for him.

So that's where we're at. I think that, now, the waiting is the hardest part. Even harder than watching Eddy have seizures. I know that he is going to have seizures until we figure out the best course of treatment for him, and I just want to have that information now.

I don't want to sound ungrateful, or negative, because I know how lucky I am to even have this little boy in my life. And I'm so fortunate to have my husband, and my family, and friends, and everything that God has given us. So many people care about Eddy, and us, and it's so comforting to know that. Please continue to pray for us, especially that God will lead us in the right direction for a doctor and a treatment that will help cure Eddy. Thanks so much <3.

The Rollercoaster Continues

Well we just got home from our latest hospital trip, this time for a 48 hour EEG, and I thought I would give an update about what's going on. Everything is just as confusing as it's been for the past two months! Nothing is clear-cut with this little boy! As you know, last week, we got good news that Eddy's spasms had basically gone away...or he didn't have any during the 24 hours he was hooked up to the monitors. Four weeks ago was the first time we were told Eddy had infantile spasms, but even then we were told that he was "atypical." He didn't have the spasms in clusters, which is typical for infantile spasms, and he only had them when he was sleeping. In addition, he only had a few of them, maybe five. (A lot of babies have lots of spasms a day, sometimes into the hundreds.) Also, the spasms turned into tonic seizures, which isn't the typical "passway" of seizures. (Normally a baby would have a spasm, have another spasm, have another spasm, etc. Eddy would start with a short spasm, but it was turning ino a tonic seizure, where his arms went rigid for 5-10 seconds.) So, four weeks ago, we were all set to start ACTH, a horrible steroid treatment that is recommeded first a lot of the time for infantile spasms because it has the highest success rate of making everything go away. Then Ed got sick and we decided to wait a couple of weeks and see if Eddy was still going to need ACTH.

Then Eddy seemed to be doing better, not having a seizure for two and a half weeks. And his development was improving by leaps and bounds. But then, about two weeks ago, he started having seizures again. He had three last week, and he had a bad one last Saturday. He had another one Wednesday night when he was hooked up to the monitors (which is actually a good thing, if he's going to have them, it's better that it's when he's hooked up to the EEG). The one Wednesday was the exact same as the one last Saturday - his arms came up like a spasm, then went rigid (tonic seizure), then he started shaking and his eyes rolled (tonic/clonic, or grand mal seizure). So, Dr. Weinstock was very worried yesterday morning when he spoke with us, because the spasms were back, and one of the two turned into a grand mal seizure. He said that we had to start a treatment, and we had to decide by today what that treatment would be. He recommended ACTH again but said we could also try prednisolone (a steroid similar to ACTH but not as harsh) or vigabatrin (which can cause irreversible damage to your eyes). So all day yesterday we discussed each treatment and tried to decide what to do. We were leaning towards ACTH, but were waiting to talk to Dr. Weinstock again this morning, after he reviewed Eddy's EEG from last night.

So this morning, Dr. Weinstock came in the room and said that Eddy didn't have any spasms or seizure activity last night. He stated once again that Eddy is very atypical, and he does not fit into any "standard case." He said that, a few weeks ago, Eddy had him very very worried about the spasms and seizures, and he knew we had to start a treatment. Then Eddy seemed to get better and he felt good about his progress. Last week, he seemed more optimistic about Eddy's EEG. Then yesterday he was again very worried because of the seizures and spasms, and he knew we had to start a treatment. But today he said that Eddy didn't have any seizure activity during the night, and so we can hold off for now. We are just going to increase Eddy's Keppra to the max dosage and see if that does anything to stop the seizures he's still having. It's such a rollercoaster!!

I guess the bottom line is that Eddy is a very special little boy who is not going to fit into any mold! On the one hand, we are very happy that we don't have to be in the hospital all week next week and put Eddy through the horrible treatment of ACTH. On the other hand, Eddy is still having the weird, abnormal EEG brain waves when he sleeps...and he's still having seizures...so chances are, the spasms are going to come back, and we might end up having to do one of the three above-mentioned treatments after all. Everything is just so up in the air right now, and we have to constantly be on our toes. I feel like I should just get a device that attaches from my head to the video monitor so I don't have to keep carrying it around. I probably look at it every ten seconds when I'm awake, and every fifteen minutes during the night. Occasionally, I'll sleep for an hour or two, but I'll wake up terrified that I missed something. I know that this won't be the case forever (well I'm hoping it's not!), so I just have to be strong and alert for right now. And tomorrow my fabulous parents are spending the night and taking the video monitor for me once again so I can get some sleep. (Thanks mom and dad, you are the best!!)

I wish you all could have seen Eddy when we got home this afternoon. He was so happy that he was finally able to move around that he just crawled around in big circles, playing with his toys, for a half hour. It was adorable. I was worried that not being able to practice any of his new skills for the last couple days might hurt his development, but he was crawling faster than I've ever seen him and pulling up on the couch like a champ. So there are lots of positive, encouraging things going on with Eddy, and these are the things I need to focus on.

Another positive is that my wonderful husband made my birthday very special with roses, playing our wedding song, and treating me to a great birthday dinner at the Hostaga Restaurant in Olean. It was the perfect, relaxing end to a stressful week, and it gave us a refreshed, new outlook on everything.

Thanks for the prayers and birthday wishes - you are great friends and family and I really appreciate you all!